The patient hadn’t eaten anything substantial for years due to nausea so constant that she couldn’t keep down solid food. Instead, she “ate” daily through an IV line. When asked what food she missed she said none: The very thought made her sick to her stomach.
Then there was the dizziness and the mysterious fevers — spiking to 106 degrees Fahrenheit at times — caused by bloodstream infections that struck without warning and that, when the doorbell rang, made her wonder whether she would make it through an otherwise routine visit with friends.
Now 23, she felt like it was an eternity ago when she was healthy and 15, playing soccer and running track. As her symptoms appeared and worsened, doctors near her home in Springfield were baffled. Her body seemed to be attacking itself, but tests for known autoimmune conditions came back negative. The standard options were quickly shrinking and solving the puzzle would ultimately require an innovative, highly individualized approach.
In 2018, in desperation, the family agreed to an autologous blood stem cell transplant at a Chicago hospital. The transplant is a radical procedure that begins with the extraction of blood stem cells from bone marrow, presumably the abnormality’s roots, which is then treated with chemotherapy to get rid of the problem cells. Once completed, the collected stem cells were infused to repopulate her bone marrow, rejuvenate her blood and, hopefully, reboot her immune system. But her symptoms only got worse.
“The arc of her infections seemed to increase after the stem cell transplant,” said Ryan Thompson, an assistant professor of medicine at Harvard Medical School and a member of Massachusetts General Hospital’s inpatient Complex Care Service. “She has had a 180-degree change in her life since the initial onset of her illness.”
Thompson met the young woman, Emily Hedspeth, in August 2019, months before the coronavirus pandemic sent the world tumbling into chaos. She had come to MGH after her symptoms worsened following the stem cell transplant, and Thompson’s team was called in.
The Complex Care Service was created for patients like Hedspeth who are, as Thompson described, “the sickest 1 percent of the sickest 1 percent.” They are patients whose conditions ensure frequent hospitalizations and who otherwise might see different physicians and nurses on each visit. Instead, they are assigned a team that follows them over time, providing continuity in their care, allowing team members to gain experience with that patient’s particular case, and building trust with the patient and family.
After evaluating her in consultation with specialists, Thompson said, there seemed to be two issues at play in the young woman’s body. First, her gut lining was leaky, allowing bacteria and fungi into her bloodstream, where they caused infection. Second, her immune system seemed to be impaired and unable to fight off that infection. Even with a healthy gut, pathogens find their way into the bloodstream from time to time, Thompson said. Though that provided a starting point, they didn’t know the cause of each problem.
Hedspeth went home after being treated but, as feared, soon returned. Her second visit proved a marathon, stretching from days to weeks to months as physicians tried to tamp down the infections to the point where she could go home. After years of struggling with the condition, it was sometimes hard for Hedspeth to keep up her spirits.
“There have been some times when she has been hopeless,” said Priscilla Parris, a nurse practitioner on the Complex Care Service who works with Hedspeth. “We met her at the beginning of her steepest decline. There was a lot of anxiety at home not knowing when an infection would hit.”
Parris said Hedspeth is able to move around, but lives with a malaise, constantly fatigued and nauseous. A bright spot during that lengthy stay — it would stretch to three months — came compliments of something many young adults would rather skip: a test. Hedspeth had completed work toward her nursing certificate in May 2019, before entering MGH, but had missed the certification board exams. She worked with Parris to bone up, then took and passed the exam.
“I quizzed her a little,” Parris said. “We got her out to take the boards.”
As it became clearer that an immune dysfunction was at work, Thompson called a friend from residency, Michael Mansour, who had an MGH lab focused on neutrophils, the most common immune cell in the bloodstream and a prime candidate for Hedspeth’s immune dysfunction.